Primary pulmonary sarcomas (PPSs) are a rare group of pulmonary malignancies, outnumbered by bronchogenic carcinomas by a factor of 500:1. These tumors are often difficult to differentiate and characterize on the basis of morphology alone, and diagnosis frequently depends on immunohistochemical and ultrastructural analysis. PPSs are typically very advanced at the time of diagnosis, with symptoms relating to tumor invasion or postobstructive pneumonia commonly prompting evaluation. Some patients, however, are asymptomatic at the time of diagnosis, with an incidental finding on an imaging study. Clinical symptoms and radiographic findings can resemble a diverse collection of other disease entities; thus, PPS needs to be entertained in the differential diagnosis in the evaluation of other diseases. Complete surgical resection remains the only approach for cure, although many patients are not eligible for resection at the time of diagnosis. Chemotherapy and radiation therapy play only limited roles in treatment, and newer, more efficacious, therapeutic agents are needed.
- Epithelioid hemangioendothelioma
- Kaposi's sarcoma
- Malignant fibrous histiocytoma
- Pulmonary sarcoma
- Synovial sarcoma
ASJC Scopus subject areas